Castleman变异型POEMS综合征

Russell H, et al, Radiographics. 2011;31(6):1793-1807.
a There is a Castleman disease variant of POEMS syndrome that occurs without evidence of a clonal PCD that is not accounted for in this table. This entity should be considered separately.
Relationship to Castleman Disease and Castleman Disease Variant of POEMS
• Castleman Disease (or angiofollicular lymph node hyperplasia) is a rare lymphoproliferative disorder, which has many presentations, ranging from an asymptomatic unifocal mass to multifocal masses with a multitude of symptoms. • The symptoms can range from simple β-symptoms to various autoimmune phenomenon to a frank POEMS syndrome (Fig. 1)
Castleman B, et al, N Engl J Med. 1954;250(1):26-30. Castleman B, et al, Cancer. 1956;9(4):822-830. Keller AR, et al, Cancer. 1972;29(3):670-683. Herrada J, et al, Ann Intern Med. 1998;128(8):657-662.
Developing other diseases
• Although Castleman disease is not a malignant condition, the condition has been associated with an increased risk of developing certain malignancies and other diseases, most notably large B-cell lymphomas, along with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin abnormality (POEMS) syndrome, follicular dendritic cell sarcomas, and paraneoplastic pemphigus.
Castleman变异型POEMS综合征
Castleman disease
• Castleman disease, also known as angiofollicular lymph node hyperplasia, is originally described in a case published in 1954, is an uncommon lymphoproliferative disorder characterized as either unicentric or multicentric. • Histologically, Castleman disease may be classified as either the hyaline-vascular or plasma cell variant, with occasional cases demonstrating mixed features.
Date back
• Several published cases of “interesting features” associated with Castleman disease are likely cases of POEMS syndrome.
Gaba AR, et al, Am J Clin Pathol 1978;69:86–90. Hineman VL, et al, Mayo Clin Proc 1982;57:379–382. Black DA, et al, Postgrad Med J 1988;64:217–219. Feigert JM, et al, Ann Intern Med 1990;113:362–367.
• There is a Castleman disease variant of POEMS syndrome may is associated with a clonal plasma cell disorder (PCD). • POEMS syndrome should be distinguished from the Castleman disease variant of POEMS syndrome, which has no clonal PCD and typically little to no peripheral neuropathy but has several of the minor diagnostic criteria for POEMS syndrome.
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Scott D, et al, Am J Hematol. 2001;66(2):148-150. Oksenhendler E, et al, Blood. 2002;99(7):2331-2336. Bélec L, et al, Blood. 1999;93(11):3643-3653.
Disease Overview